Information on all the types of lymphangiectasia, including intestinal, pulmonary, renal, cutaneous (skin). Sponsored by Pat O'Connor

Wednesday, November 29, 2006

Long-term follow-up of peripelvic renal multicystic lymphangiectasia

Long-term follow-up of peripelvic renal multicystic lymphangiectasia

[Article in Italian]
Battaglia M,
Ditonno P,
Mancini V,
Stellacci V,
Bettocchi C,
Palazzo S,
Martino P,
Selvaggi FP.

Sezione di Nefrologia Chirurgica e Trapianto, Dipartimento dell'Emergenza e dei Trapianti d'Organo, Universita degli Studi di Bari.

Renal peripelvic multicystic lymphangiectasia is a benign disease characterized by multiple cysts arising from the lymphatic vessels of the renal sinus. Cysts, almost always bilateral, surround the profiles of the calices; the biggest cysts can compress pelvis or iuxtapielic ureter and it is difficult to differentiate RPML from hydronephrosis at the Ultrasound. Cysts are asymptomatic, the profiles of calices appear irregular with thin membrane separating each other, the cortical of the kidney is preserved: these are principle elements to distinguish RPML to hydronephrosis at the Ultrasound. Usually it is necessary to confirm the diagnosis at IVP and CT scan evaluation.

From 1995 to March 2002, 10 cases of RPML with a long-term follow-up have been studied. IVP in 8 patients and CT scan in 2 have shown compression of the collecting system by multiple cysts. We followed the cases with periodic lab tests of kidney function, ultrasound, IVP, CT scan. In order to valuate the presence of "true obstruction", we tested in over-night urine EGF and MCP-1 as markers of urinary tract obstruction and subsequent renal damage. A valuable data regarding how to differentiate RPML from hydronephrosis at Ultrasound show that RPML does not modify renal function and cyst volume has not changed in the time.

In long-term follow-up the prognosis of the RPML is not clear. Particolarly this renal sinus disease has not neoplastic degeneration and the effect of cysts on kidney function is unknown. RPML is an uncommon disease and it can be enclosed in the group of renal sinus pathologies. The absence of symptoms, the hystologic diagnosis, the unchanged dimensions of the cysts confirm the benign prognosis of RPML which does not need a close functional and morphologic monitoring in the long-term follow-up.

PMID: 12508731 [PubMed - indexed for MEDLINE]

Tuesday, November 07, 2006

Congenital pulmonary lymphangiectasia (PL)

Congenital pulmonary lymphangiectasia (PL)
Orphanet J Rare Dis. 2006 Oct 30;1(1):43 [Epub ahead of print] Bellini C, Boccardo F, Campisi C, Bonioli E.


Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung and characterized by pulmonary subpleural, interlobar, perivascular, and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion.

The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress.

Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.